UNASSIGNED: This study aims to elucidate the clinical features observed in cases of pediatric acute myeloid leukemia (AML) initially presenting with cardiac tamponade and to share treatment experiences.
UNASSIGNED: Five pediatric patients were initially diagnosed with AML accompanied by cardiac myeloid sarcoma (MS). The diagnosis was established by examining our hospital records and reviewing pertinent literature from 1990 to July 2023, accessible through MEDLINE/PubMed. We comprehensively assessed the clinical characteristics and treatment modalities employed for these patients.
UNASSIGNED: Five pediatric patients presented with acute symptoms, including shortness of breath, malaise, cough, and fever, leading to their hospitalization. Physical examination revealed irritability, hypoxia, tachypnea, tachycardia, and hypotension. Initial detection utilized chest X-ray or echocardiogram, leading to subsequent diagnoses based on pericardial effusion and/or bone marrow examination. Two patients received chemotherapy at the time of initial diagnosis, one with cytarabine and etoposide, and the other with cytarabine and cladribine. Post-treatment, their bone marrow achieved remission, and over a 2.5-year follow-up, their cardiac function remained favorable. Unfortunately, the remaining three patients succumbed within two weeks after diagnosis, either due to receiving alternative drugs or without undergoing chemotherapy.
UNASSIGNED: This is the first and largest case series of pediatric AML patients with cardiac MS, manifesting initially with cardiac tamponade. It highlights the rarity and high mortality associated with this condition. The critical factors for reducing mortality include identifying clinical manifestations, conducting thorough physical examinations, performing echocardiography promptly, initiating early and timely pericardial drainage, and avoiding cardiotoxic chemotherapy medications.

UNASSIGNED: The controlling nutritional status score (CONUT) has been widely used for ascertaining the prognosis of various cancers. However, its use in patients with hematological malignancies remains unclear. This review examined evidence on the utility of CONUT as a prognostic marker for patients with hematological malignancies.
UNASSIGNED: All cohort studies that examined the association between CONUT and outcomes of hematological malignancies and were published on the databases of Embase, Scopus, CENTRAL, Web of Science, and PubMed were searched from the inception of the databases to 30 January 2024. The primary outcome was overall survival (OS), and the secondary outcome was progression-free survival (PFS).
UNASSIGNED: A total of 23 studies were available for review. A meta-analysis of 22 studies showed that high CONUT was significantly associated with poor OS in patients with hematological malignancies (HR: 1.95 95% CI: 1.62, 2.35 I 2 = 89%). The results remained unchanged on sensitivity and subgroup analyses based on study location, sample size, diagnosis, CONUT cutoff, and the Newcastle-Ottawa Scale score. Only six studies reported data on PFS, and the pooled analysis found that high CONUT was a significant marker for poor PFS in patients with hematological malignancies [hazards ratio (HR): 1.64 95% CI: 1.21, 2.20 I 2 = 70%]. These results, too, maintained significance in the sensitivity analysis.
UNASSIGNED: CONUT is an independent predictor of poor OS in patients with hematological malignancies. The results appear to be valid across different cancer types and with different CONUT cutoffs. Scarce data also suggest that CONUT could predict PFS.

In recent years, the importance of long non-coding RNA (lncRNA) in acute myeloid leukemia (AML) has attracted wide attention. Among them, lncRNAs that play a role in promoting cancer mainly include HOTAIR, UCA1, H19, ITGB2-AS1 and some genes of SNHG family, while in tumor suppression mainly include H22954, NEAT1, SNHG4, LINC01128 , etc. This article reviews the role of lncRNAs in the occurrence and development of AML, as well as those related to AML resistance and prognosis assessment, so as to provide a theoretical basis for the diagnosis and prognosis analysis of AML.
UNASSIGNED: 长链非编码RNA在急性髓细胞白血病发生发展中作用的研究进展.
UNASSIGNED: 近年来，长链非编码RNA（lncRNA）在急性髓细胞白血病（AML）中的重要性引起人们的广泛关注，其中发挥促癌作用的lncRNA主要有HOTAIR、UCA1、H19、ITGB2-AS1 和SNHG 家族的部分基因，而起抑癌作用的lncRNA主要有H22954、NEAT1、SNHG4、LINC01128等。本文就lncRNA在AML发生发展中的作用以及与AML耐药和预后判断相关的lncRNA进行综述，以期为AML的诊断和预后分析提供理论依据。 .

Chimeric antigen receptor (CAR) T cell therapy, one of the most promising tumor treatments, combines the targeted recognition of antigen and antibody with the killing effect of T cells. CAR-T has shown a strong therapeutic effect in lymphoid tumors and been applied in clinical practice. However, in the treatment of acute myeloid leukemia (AML), no effective and specific target like CD19 in lymphoid tumors has been found. Therefore, the key research direction is to try multiple probabilities and use optimization strategies to enhance efficacy and reduce toxicity. This review introduces the latest research progress of AML targets in CAR-T therapy in recent years, analyzes the related problems that need to be solved at present, and summarizes the optimization construction strategies mentioned in the research. Hope it can provide reference for related research and clinical application of related product.
UNASSIGNED: 急性髓系白血病CAR-T治疗的靶点选择.
UNASSIGNED: 嵌合抗原受体（CAR）T细胞疗法将抗原抗体的靶向识别与T细胞的杀伤作用相结合，已成为最有应用前景的肿瘤治疗方法之一。在淋巴系肿瘤中，CAR-T显示出了强大的治疗效果，并已陆续在临床中得到应用。然而，在急性髓系白血病的治疗中尚未发现如淋巴系肿瘤中CD19一样有效且特异的靶点，因此，尝试多种靶点，并且使用优化策略，以增强疗效、减轻毒性，是目前主要的研究方向。本文介绍了近年来CAR-T治疗急性髓系白血病靶点的最新研究进展，分析了目前亟待解决的相关问题，并总结了研究中提到的优化构建策略，希望能为相关研究的进行以及产品的临床应用提供参考。.

Richter transformation (RT) represents the development of intrusive lymphoma in individuals previously or concurrently diagnosed with chronic lymphocytic leukemia (CLL) and is characterized by lymph node enlargement. However, cases involving extra-nodal organ involvement as the first symptom are rare. There are no reports of RT with breast lesions as the first symptom. Nonspecific and atypical clinical manifestations represent key challenges in the accurate diagnosis and appropriate treatment of RT. This case report describes an elderly female patient who presented with breast lesions as the first RT symptom. The patient was admitted with a painless mass in the left breast. Examination revealed multiple lymphadenopathies and abnormally high white blood cell levels. The patient was diagnosed with CLL after hematological tests, assessments of bone marrow morphology, and tissue biopsy. Mammography and B-ultrasonography showed solid space-occupying lesions (BI-RADS category 5) in the left breast. Initially, the patient declined a breast biopsy and was therefore prescribed ibrupotinib treatment, which showed limited efficacy. A needle biopsy of the affected breast indicated the presence of diffuse large B-cell lymphoma. Based on auxiliary and pathological examinations and medical history, the final diagnosis was RT with breast involvement. Zanubrutinib with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone treatment provided initial control; however, the treatment strategy required adjustment because of the patient\'s fluctuating condition. The current status of the patient is marked as stable, showing an overall achievement of partial alleviation. The patient is in the process of receiving follow-up treatment. We also performed a comprehensive literature review on RT, with particular emphasis on its biological paradigm, prognosis implications, existing therapeutic approaches, and emerging directions in treatment modalities.

Acute leukemias (ALs) are the most common cancers in pediatric population. There are two types of ALs: acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). Some studies suggest that the Renin Angiotensin System (RAS) has a role in ALs. RAS signaling modulates, directly and indirectly, cellular activity in different cancers, affecting tumor cells and angiogenesis. Our review aimed to summarize the role of RAS in ALs and to explore future perspectives for the treatment of these hematological malignancies by modulating RAS molecules. The database including Pubmed, Scopus, Cochrane Library, and Scielo were searched to find articles about RAS molecules in ALL and in pediatric patients. The search terms were \"RAS\", \"Acute Leukemia\", \"ALL\", \"Angiotensin-(1-7)\", \"Pediatric\", \"Cancer\", \"Angiotensin II\", \"AML\". In the bone marrow, RAS has been found to play a key role in blood cell formation, affecting several processes including apoptosis, cell proliferation, mobilization, intracellular signaling, angiogenesis, fibrosis, and inflammation. Local tissue RAS modulates tumor growth and metastasis through autocrine and paracrine actions. RAS mainly acts via two molecules, Angiotensin II (Ang II) and Angiotensin (1-7) [Ang-(1-7)]. While Ang II promotes tumor cell growth and stimulates angiogenesis, Ang-(1-7) inhibits the proliferation of neoplastic cells and the angiogenesis, suggesting a potential therapeutic role of this molecule in ALL. The interaction between ALs and RAS reveals a complex network of molecules that can affect the hematopoiesis and the development of hematological cancers. Understanding these interactions could pave the way for innovative therapeutic approaches targeting RAS components.

Intermittent fasting (IF) has recently gained popularity due to its emerging benefits in reducing weight and improving metabolic health. Concurrently, novel agents (NAs) like venetoclax and Bruton tyrosine kinase inhibitors (BTKIs) have revolutionized the treatment of chronic lymphocytic leukemia (CLL). Unfortunately, it is unclear whether the associated risks of tumor lysis syndrome (TLS) and gastrointestinal bleeding (GIB) are increased in IF practitioners receiving NAs. This review explored the literature available on the permissibility of IF in CLL patients undergoing treatment with first-line NAs (FLNAs). Literature was scoped to identify IF patterns and the available data on TLS and GIB risks associated with food and fluid intake in CLL patients receiving FLNAs. Although current evidence is insufficient to recommend IF in this population, it may be possible for patients on venetoclax to conservatively practice fluid-liberal IF, provided that adequate hydration and the consistent administration of food are achieved. In contrast, considering the significant risk of TLS and the pharmacokinetics of venetoclax, patients should be discouraged from practicing fluid-restricted IF, especially during the ramp-up phase. Moreover, patients on BTKIs ought to refrain from IF due to the possible risk of GIB until further data are available. Further research is needed to provide conclusive recommendations.

BACKGROUND: Acute myeloid leukemia (AML) is aggressive type of hematological malignancy. Its poses challenges in early diagnosis, necessitating the identification of an effective biomarker. This study aims to assess the diagnostic accuracy of long noncoding RNAs (lncRNA) in the diagnosis of AML through a meta-analysis. The study is registered on the PROSPERO website with the number 493518.
METHODS: A literature search was conducted in the PubMed, Embase, Hinari, and the Scopus databases to identify relevant studies. We pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio (DOR), and area under the summary receiver operating characteristics (ROC) using Stata 14.1 software. Heterogeneity between studies was determined through the I2 statistic and Cochran-Q test. A random effect model was chosen due to significant heterogeneity among included studies. Meta-regression and subgroup analysis were performed to assess the potential source of heterogeneity. Furthermore, potential publication bias was estimated using Deek\'s funnel plot asymmetry test.
RESULTS: A total of 14 articles covering 19 studies were included in this meta-analysis comprising 1588 AML patients and 529 healthy participants. The overall pooled sensitivity, specificity, PLR, NLR, DOR, and the area under the summary ROC curve were 0.85 (95% CI = 0.78-0.91), 0.82 (95% CI = 0.72-0.89), 4.7 (95% CI = 2.9-7.4), 0.18 (95% CI = 0.12-0.28), 26 (95% CI = 12-53), and 0.90 (95% CI = 0.87-0.93), respectively. Moreover, lncRNAs from non-bone marrow mononuclear cells (BMMC) had superior diagnostic value with pooled sensitivity, specificity, and AUC were 0.93, 0.82, and 0.95, respectively.
CONCLUSIONS: This meta-analysis demonstrated that circulating lncRNAs can serve as potential diagnostic markers for AML. High accuracy of diagnosis was observed in non-BMMC lncRNAs, given cutoff value, and the GADPH internal reference gene used. However, further studies with large sample size are required to confirm our results.

UNASSIGNED: Although genetic mutations in additional sex-combs-like 1 (ASXL1) are prevalent in acute myeloid leukemia (AML), their exact impact on the AML prognosis remains uncertain. Hence, the present article was carried out to explore the prognostic importance of ASXL1 mutations in AML.
UNASSIGNED: We thoroughly searched electronic scientific databases to find eligible papers. Twenty-seven studies with an overall number of 8,953 participants were selected for the current systematic review. The hazard ratio (HR) and 95% confidence interval (CI) for overall survival (OS), event-free survival (EFS), and relapse-free survival (RFS) were extracted from all studies with multivariate or univariate analysis. Pooled HRs and p-values were also calculated as a part of our work.
UNASSIGNED: The pooled HR for OS in multivariable analysis indicated that ASXL1 significantly diminished survival in AML patients (pooled HR: 1.67; 95% CI: 1.342-2.091).
UNASSIGNED: ASXL1 mutations may confer a poor prognosis in AML. Hence, they may be regarded as potential prognostic factors. However, more detailed studies with different ASXL1 mutations are suggested to shed light on this issue.

BACKGROUND: All-trans retinoic acid (ATRA) is an indispensable part of the treatment of acute promyelocytic leukemia (APL). Although, mild cutaneous toxicities like mucocutaneous xerosis, rash, and pruritus are well reported, ATRA associated severe dermatological toxicities are extremely rare. ATRA is primary metabolized by cytochrome P450 (CYP450) enzyme system, and triazole antifungals are notorious for their strong inhibitory effect on CYP450.
METHODS: Three Asian APL patients experienced rare ATRA-induced severe dermatological toxicities: exfoliative dermatitis (ED) in cases 1 and 2, and necrotic scrotal ulceration in case 3. Both case 1 (33-year-old female), and case 2 (28-year-old male) landed in emergency department with dehydration, generalized skin erythema and xerosis during their induction chemotherapy. Both of these patients also developed invasive aspergillosis and required concomitant triazole antifungals during their chemotherapy. For ED, intravenous fluids and broad-spectrum antibiotics were started along with application of local emollients to prevent transdermal water loss. Although their general condition improved but skin exfoliation continued with complete desquamation of palms and soles. Dermatology was consulted, and clinical diagnosis of ED was established. Discontinuation of ATRA resulted in complete resolution of ED. Case 3 (15-year-old boy) reported two blackish mildly tender scrotal lesions during induction chemotherapy. He also had mucocutaneous candidiasis at presentation and was kept on triazole antifungal. Local bacterial & fungal cultures, and serological testing for herpes simplex virus were reported negative. Despite adequate local care and optimal antibiotic support, his lesions persisted, and improved only after temporary discontinuation of ATRA. After a thorough literature review and considering the temporal association of cutaneous toxicities with triazole antifungals, we speculate that the concomitant use of triazole antifungals inhibited the hepatic metabolism of ATRA, resulting in higher serum ATRA concentration, and markedly accentuated cutaneous toxicities in our patients.
CONCLUSIONS: By highlighting this crucial pharmacokinetic interaction, we want to caution the fellow oncologists to be mindful of the inhibitory effect of triazole antifungals on CYP450. We propose using a non-myelosuppressive combination of ATRA and arsenic trioxide for management of APL hence, obliterating the need of prophylactic antifungals. However, in the event of invasive fungal infection (IFI), we suggest using alternative class of antifungals.